The role of the pharmacist in medication management

The pharmacist’s role in treating sickle cell disease revolves around appropriate pain management.

In 2008, the United Nations declared June 19 as World Sickle Cell Day with the aim of raising awareness internationally of a common genetic disease, sickle cell disease (SCD). Although millions of people worldwide have SCD, there are currently approximately 100,000 Americans who have been diagnosed and approximately 1 in 13 African Americans carry the sickle cell trait.1.2

In particular, the pharmacist’s role in SCD management revolves around appropriate pain management, as the disease is notorious for its episodes of pain, and prophylactic therapy for sickle cell crisis. SCD manifests due to a single base substitution found in the beta-globin gene of chromosome 11, resulting in valine replacing glutamic acid, producing sickled hemoglobin S instead of the typical disc-shaped hemoglobin A.3

The altered form subsequently triggers the vaso-occlusive events (sickle cell crisis) that lead to acute and chronic pain in these patients. Because pain is so subjective and variable in these patients, it complicates management in this population, allowing pharmacists to intervene to optimize treatment regimens.

In acute treatment, pharmacists can ensure appropriate dosing, medication, and routes of administration as these patients have typically developed drug tolerance requiring higher doses of intravenous pure opioids and their hypoxemic condition combined with poor venous access suggest that these Patients may benefit more from oral or subcutaneous administration.3

After acute treatment, pharmacists can be critical in managing these patients’ chronic pain medications, ensuring they are effectively managing the pain while using the safest long-term option. Some examples include ensuring that patients taper opioid treatment after acute attacks to avoid withdrawal symptoms, regular monitoring of liver and kidney function for possible debilitation due to compensation for hematological abnormalities to ensure appropriate dosing, use of adjuvants for supplemental relief of symptoms, and management of long-term use of opioids, such as B. Constipation.3

In addition, as the opioid crisis is making access to these necessary medicines significantly more difficult for this patient population, pharmacists should serve as advocates for increased access, as well as educators for reluctant prescribers.4 Treatment of SCD should generally include measures to prevent sickle cell crisis.

Because of its ability to prevent vaso-occlusive events, hydroxyurea is the main treatment option to prevent pain episodes, acute chest syndrome hospitalizations, and blood transfusions.3 Treatment with hydroxyurea should be considered in patients with 3 or more episodes of pain in one year.

The pharmacist should confirm that dosages are optimized based on weight, age, hemoglobin F levels, and degree of myelosuppression.3 Adherence with hydroxyurea can be challenging in some patients.3

Pharmacists can help improve adherence to therapy through a variety of traditional methods. Routine in-hospital counseling after initiating hydroxyurea treatment about the importance of drug adherence for optimal outcomes and education about what to practically expect based on the drug’s side effect profile can better understand treatment expectations and hopefully improve adherence rates.

This, in turn, may help reduce sickle cell crises and subsequent hospitalizations and/or opioid use. Because hydroxyurea is classified as Pregnancy Category D, all initiating patients should be counseled to other healthcare providers and potentially pregnant women about the risks of this drug to fetal development, and to discuss other treatment options after stopping hydroxyurea.

Given the age demographics of SCD, which includes more patients younger in age than many common comorbidities, recognizing the risk in pregnancy is crucial. Although there is some literature that provides more optimistic data on hydroxyurea in pregnancy, the severity of the teratogenic effects, particularly in early pregnancy, is so profound that some experts recommend stopping the drug before the patient becomes pregnant.5

On the other hand, hydroxyurea therapy can be considered on rare occasions during the second and third trimesters when the risk of not taking the drug is thought to significantly outweigh the risk to the baby.4

More recently, in 2017, the FDA approved an add-on prevention drug, Endari (oral L-glutamine powder), for SCD patients over the age of 5, which can be added to hydroxyurea to prevent sickle cell crises.3 Although pharmacist advice and management are similar to hydroxyurea, administration advice is more important as the drug needs to be taken twice a day and dissolved in a liquid.

Endari is much more expensive than hydroxyurea, which makes access to this drug much more difficult. Because SCD patients, even with private insurance, may incur significant out-of-pocket costs for their care, pharmacists can play a large role in advocating for improved affordability of care.6

While pharmacological treatment is essential in the management of SCD pain and preventative options, non-pharmacological treatment also plays a key role in this patient population. Some examples of this are cognitive behavioral therapy, massage therapy, acupuncture, participation in social support groups.7

In all patient care settings, pharmacists can consult and engage with patients with resources to improve and prevent severe pain episodes. In addition, pharmacists are optimally placed to provide patient-specific advice to parents of young people diagnosed with SCD, as treatment can be difficult.

SCD is one of the most common genetic diseases in the world. Because treatment options are minimal and adherence rates are typically low, there is a clear need for interventions and improvements in the management of this patient population.

Pharmacists can fill these gaps by increasing patient counseling on preventive medications, managing acute and chronic pain therapies, and, arguably most importantly, providing thorough education to patients, caregivers and other members of the healthcare team.

references

  1. World Sickle Cell Day – Friday 19 June 2020 // Sickle Cell Society. sickle cell society. (2021, October 20). Retrieved May 15, 2022 from https://www.sicklecellsociety.org/wscd/
  2. home. Sickle Cell Disease Association of America Inc. (nd). Retrieved May 15, 2022 from https://www.sicklecelldisease.org/
  3. Ikechukwu Okwerekwu, PDC (2018, August 17). Pain therapy in sickle cell anemia. US Pharmacist – The leading journal for pharmacy. Retrieved May 15, 2022 from https://www.uspharmacist.com/article/sickle-cell-disease-pain-management
  4. The opioid crisis increases the pain of sickle cell patients. National Heart, Lung and Blood Institute. (2017, September 15). Retrieved May 20, 2022 from https://www.nhlbi.nih.gov/news/2017/opioid-crisis-adds-pain-sickle-cell-patients
  5. R. Montironi, R. Cupaiolo, C. Kadji et al. Management of sickle cell disease during pregnancy: experiences in a third-level hospital and future recommendations. J Matern Fetal Neonatal Med.2020:1-3
  6. The cost of living in sickle cell anemia. American Society of Hematology. (2022, May 16). Retrieved May 20, 2022 from https://www.hematology.org/newsroom/press-releases/2022/the-cost-of-living-with-sickle-cell-disease
  7. Williams PhD, H. (nd). Sickle Cell Disease: A Review of Non-Pharmacological Approaches to Pain: Review Articles . Journal of Pain and Symptom Management. Retrieved May 15, 2022 from https://www.jpsmjournal.com/article/S0885-3924(15)00606-5/pdf

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